Analyzing fatal cases of Chinese patients with primary antineutrophil cytoplasmic antibodies-associated renal vasculitis: a 10-year retrospective study.
OBJECTIVE
Primary antineutrophil cytoplasmic antibodies (ANCA)-associated systemic vasculitis (AASV) used to have poor prognosis, and renal involvement is its most common manifestation. Few studies have been published focusing on AASV patients with poor prognosis.


METHODS
From 1997 to 2006, 101 patients with ANCA-associated renal vasculitis (70 microscopic polyangiitis, MPA; 14 Wegener's granulomatosis, WG; 3 Churg-Strauss syndrome, CSS; 14 renal limited vasculitis, RLV) were diagnosed in Shanghai Ruijin Hospital and 26 deaths were recorded among them. Patients' data were retrospectively analyzed.


RESULTS
Patients with WG, MPA and RLV made up for 23.1% (6/26), 65.4% (17/26) and 11.5% (3/26) of all deaths. No deaths were observed among CSS patients. Infection alone accounted for 13 deaths. Infection together with pulmonary involvement of active vasculitis accounted for 3. Organ-specific involvement of active vasculitis alone caused 8 deaths. Others died of acute myocardial infarction or gastric carcinoma. Compared with patients who survived, nonsurvivors had more severe renal insufficiency and older age (p < 0.01). There was no significant difference regarding clinical presentation at diagnosis and cause of death between patients who survived first remission-induction treatment and those who did not. Infection remained the major cause of death.


CONCLUSIONS
Infection is the major cause of death in patients with ANCA-associated renal vasculitis, and treatment response might not correlate to severity of disease in patients with poor prognosis. Rational use of immunosuppressants could improve the prognosis.
Chen
Yong-Xi
YX
Department of Nephrology, Shanghai Ruijin Hospital affiliated to Shanghai Jiaotong University, School of Medicine, Shanghai, PR China.
Yu
Hai-Jin
HJ
Zhang
Wen
W
Ren
Hong
H
Chen
Xiao-Nong
XN
Shen
Ping-Yan
PY
Xu
Yao-Wen
YW
Li
Xiao
X
Pan
Xiao-Xia
XX
Ni
Li-Yan
LY
Wang
Wei-Ming
WM
Chen
Nan
N
D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D019268:Antibodies, Antineutrophil Cytoplasmic; D044466:Asian People; D002423:Cause of Death; D005260:Female; D006801:Humans; D007239:Infections; D007674:Kidney Diseases / Q000276:immunology* / Q000401:mortality / Q000628:therapy; D008297:Male; D008875:Middle Aged; D051437:Renal Insufficiency; D012189:Retrospective Studies; D014657:Vasculitis / Q000276:immunology* / Q000401:mortality* / Q000628:therapy
D019268:Antibodies, Antineutrophil Cytoplasmic